Ilaris® (Canakinumab)

Ilaris® (canakinumab)

When requesting Ilaris® (canakinumab), the individual requiring treatment must be diagnosed with an FDA-approved indication and meet the specific coverage guidelines and applicable safety criteria for the covered indication.

FDA-Approved Indications

Ilaris is indicated for the treatment of: • Cryopyrin-Associated Periodic Syndromes (CAPS), in patients 4 years of age and older • Tumor Necrosis Factor (TNF) Receptor Associated Periodic Syndrome (TRAPS) • Hyperimmunoglobulin D (Hyper-IgD) Syndrome (HIDS)/Mevalonate Kinase Deficiency (MKD) • Familial Mediterranean Fever (FMF) • Active Systemic Juvenile Idiopathic Arthritis (sJIA) in patients 2 years of age and older.

Coverage Guidelines

The individual must meet the following criteria for initial authorization of select indications:

Cryopyrin-Associated Periodic Syndromes (CAPS) • An individual is 4 years of age or older; AND • Ilaris is prescribed by or in consultation with a rheumatologist, geneticist, allergist/immunologist, or dermatologist.

Familial Mediterranean Fever (FMF) • Ilaris is prescribed by or in consultation with a rheumatologist, nephrologist, geneticist, gastroenterologist, oncologist, or hematologist.

Hyperimmunoglobulin D syndrome (HIDS)/Mevalonate Kinase Deficiency (MKD) OR Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) • Ilaris is prescribed by or in consultation with a rheumatologist, nephrologist, geneticist, oncologist, or hematologist.

Systemic Juvenile Idiopathic Arthritis (sJIA) • An individual is 2 years of age or older; AND • Ilaris is prescribed by or in consultation with a rheumatologist; AND • An individual meets one of the following conditions: o Has tried at least 2 other biologic agents (e.g., tocilizumab, Kineret, abatacept, etanercept, adalimumab, infliximab); OR

o Has features of poor prognosis (e.g., arthritis of the hip, radiographic damage, active systemic disease such as fever, elevated inflammatory markers) and has tried Actemra or Kineret; OR o Has active systemic features with concerns of progression to macrophage activation syndrome (MAS) and has tried Kineret.

The individual must meet the following criteria for reauthorization (all indications): • An individual is responding to Ilaris therapy.

Approval duration (initial): 3 months (applies to CAPS and sJIA) Approval duration (initial): 4 months (applies to FMF, TRAPS, and HIDS/MKD) Approval duration (renewal): 12 months

Dosing Recommendations

Cryopyrin-Associated Periodic Syndromes (CAPS): The recommended dose is 150 mg for patients with body weight greater than 40 kg and 2 mg/kg for patients with body weight 15 to 40 kg administered subcutaneously every 8 weeks.

Tumor Necrosis Factor (TNF) receptor Associated Periodic Syndrome (TRAPS), Hyperimmunoglobulin D (Hyper-IgD) Syndrome (HIDS)/Mevalonate Kinase Deficiency (MKD), Familial Mediterranean Fever (FMF) For body weight less than or equal to 40 kg: The recommended starting dose is 2 mg/kg administered subcutaneously every 4 weeks. The dose may be increased to 4 mg/kg every 4 weeks if the clinical response is inadequate.

For body weight greater than 40 kg: The recommended starting dose is 150 mg every 4 weeks. The dose can be increased to 300 mg every 4 weeks if the clinical response is inadequate.

Systemic Juvenile Idiopathic Arthritis: The recommended dose is 4 mg/kg, for patients 2 years of age or older with body weight 7.5 kg or greater, administered subcutaneously every 4 weeks. The maximum dosage is 300mg per dose.

References

1. Ilaris® for subcutaneous injection [prescribing information]. East Hanover, NJ: Novartis Pharmaceuticals Corporation; December 2016. 2. Arcalyst® for injection [prescribing information]. Tarrytown, NY: Regeneron Pharmaceuticals Inc; September 2016. 3. Hoffman HM, Throne ML, Amar NJ, et al. Efficacy and safety of rilonacept (interleukin-1 Trap) in patients with (canakinumab) cryopyrin-associated periodic syndromes: results from two sequential placebo-controlled studies. Arthritis Rheum. ® 2008;58:2443-2452. 4. Shinkai K, McCalmont TH, Leslie KS. Cryopyrin-associated periodic syndromes and autoinflammation. Clin Exp

Dermatol. 2008;33:1-9. laris I

5. Ringold S, Weiss PF, Beukelman T, et al. 2013 update of the 2011 American College of Rheumatology recommendations for the treatment of juvenile idiopathic arthritis: recommendations for the medical therapy of children with systemic juvenile idiopathic arthritis and tuberculosis screening among children receiving biologic medications. Arthritis Rheum. 2013;65(10):2499-2512. 6. Ozen S, Hoffman HM, Frenkel J, et al. Familial Mediterranean Fever (FMF) and beyond: a new horizon. Fourth International Congress on the Systemic Autoinflammatory Diseases held in Bethesda, USA, 6-10 November 2005. Ann Rheum Dis. 2006;65(7):961-964. 7. Lachmann HJ, Kone-Paut I, Kuemmerle-Deschner JB, et al; Canakinumab in CAPS Study Group. Use of canakinumab in the cryopyrin-associated periodic syndrome. N Engl J Med. 2009;360:2416-2425. 8. Russo RA, Melo-Gomes S, Lachmann HJ, et al. Efficacy and safety of canakinumab therapy in paediatric patients with cryopyrin-associated periodic syndrome: a single-centre, real-world experience. Rheumatology (Oxford). 2014;53(4):665-670. 9. Kuemmerle-Deschner JB, Hachulla E, Cartwright R, et al. Two-year results from an open-label, multicentre, phase III study evaluating the safety and efficacy of canakinumab in patients with cryopyrin-associated periodic syndrome across different severity phenotypes. Ann Rheum Dis. 2011;70(12):2095-2102. 10. Sibley CH, Chioato A, Felix S, et al. A 24-month open-label study of canakinumab in neonatal-onset multisystem inflammatory disease. Ann Rheum Dis. 2015;74(9):1714-1719. 11. Kuemmerle-Deschner JB, Ramos E, Blank N, et al. Canakinumab (ACZ885, a fully human IgG1 anti-IL-1β mAb) induces sustained remission in pediatric patients with cryopyrin-associated periodic syndrome (CAPS). Arthritis Res Ther. 2011;13(1):R34. 12. Genetics Home Reference. US National Library of Medicine. Available at: https://ghr.nlm.nih.gov/. Accessed on October 29, 2018. Search terms: TRAPS, familial Mediterranean fever, MKD. 13. Ozen S, Demirkaya E, Erer B, et al. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis. 2016;75(4):644-651. 14. ter Haar NM, Oswald M, Jeyaratnam J, et al. Recommendations for the management of autoinflammatory diseases. Ann Rheum Dis. 2015;74(9):1636-1644. 15. Kimura Y, Morgan DeWitt E, Beukelman T, et al. Adding Canakinumab to the Childhood Arthritis and Rheumatology Research Alliance Consensus Treatment Plans for Systemic Juvenile Idiopathic Arthritis: comment on the article by DeWitt et al. Arthritis Care Res (Hoboken). 2014;66(9):1430-1431. 16. DeWitt EM, Kimura Y, Beukelman T, et al. Consensus treatment plans for new-onset systemic juvenile idiopathic arthritis. Arthritis Care Res (Hoboken). 2012;64(7):1001-1010. 17. Ruperto N, Brunner HI, Quartier P, et al. Two randomized trials of canakinumab in systemic juvenile idiopathic arthritis. N Engl J Med. 2012;367(25):2396-2406. 18. Novartis. Efficacy, safety and tolerability of ACZ885 in patients with active rheumatoid arthritis. In; ClinicalTrials.gov [Internet]. Bethesda (MD): National Library of Medicine (US). Accessed on October 29, 2018. Available from: http://www.clinicaltrials.gov/ct2/show/NCT00424346?term=acz885&rank=10. NLM Identifier: NCT00424346 19. Alten R, Gomez-Reino J, Durez P, et al. Efficacy and safety of the human anti-IL-1β monoclonal antibody canakinumab in rheumatoid arthritis: results of a 12-week, Phase II, dose-finding study. BMC Musculoskelet Disord. 2011;12:153. 20. Canakinumab (Ilaris®). In: DRUGDEX® System Micromedex. Greenwood Village, CO: Thompson Reuters (Healthcare) Inc. [Updated; November 06, 2019; Accessed April 24, 2020].

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